Steroid-dependent Patients Research Articles

Leukocytapheresis in the treatment of inflammatory bowel disease: Current position and perspectives

Therapeutic apheresis, a novel approach for immunodisorders, has been used in the last decade for the treatment of ulcerative colitis with promising result, and represents an alternative to conventional pharmacological therapy. Selective apheresis is aimed at reducing the number of circulating lymphocytes, interfering with recruitment and activation of mucosal granulocytes and macrophages, reducing cytokine and chemokine production which are thought to contribute to induction and perpetuation of inflammation. The article briefly reports indications, treatment schedule and clinical results of leukocytapheresis in ulcerative colitis. Available data for the two selective adsorption devices so far approved for clinical use (granulocyte-monocyte apheresis- Adacolumn- and leukocytapheresis-Cellsorba) are partially conflicting, and the number of controlled studies too small to draw definitive conclusions. Nonetheless apheresis definitely appears to be an effective non-conventional tool for the treatment of steroid refractory and steroid dependent UC patients with moderately active disease. The excellent safety profile of the procedure makes this approach attractive, both in adult and in pediatric patients, more so in those refractory to conventional drug therapy, who are presently treated with immunosuppressive and biological therapies.

Efficacy of antibiotic combination therapy in patients with active ulcerative colitis, including refractory or steroid‐dependent cases

We previously demonstrated that antibiotic combination therapy is effective for induction and maintenance of ulcerative colitis (UC) remission. Herein, we assessed whether antibiotic combination therapy is effective for active UC, including cases with steroid refractory or dependent disease. We enrolled 25 patients with active UC including 17 steroid-dependent or refractory cases. These patients received amoxicillin 500 mg t.i.d., tetracycline 500 mg t.i.d. and metronidazole 250 mg t.i.d. for 2 weeks as well as conventional treatment. Seven colonic segments from the appendiceal region to the rectum were scored for endoscopic activity and histology. Clinical activity indexes (CAI) were also determined. At 3 and 12 months after antibiotic treatment, CAI and endoscopic score were significantly decreased as compared to those before treatment (P < 0.001 and P < 0.05, P < 0.01, respectively). Histological scores were also significantly decreased at 12 months as compared to before treatment (P < 0.01). The clinical response rates in steroid-dependent patients were 60% and 73.3% at 3 and 12 months, respectively, while being 50% at 12 months in steroid-refractory patients. Among the 17 steroid-dependent or refractory patients, 12 (70.6%) were able to discontinue steroid therapy at 12 months. No serious drug-related toxicities were observed during the trial. This long-term follow-up study suggests 2-week antibiotic combination therapy to be effective and safe in patients with active UC including those with steroid-refractory or dependent disease.

Newly Developed Antibiotic Combination Therapy for Ulcerative Colitis: A Double-Blind Placebo-Controlled Multicenter Trial

Fusobacterium varium may contribute to ulcerative colitis (UC). We conducted a double-blind placebo-controlled multicenter trial to determine whether antibiotic combination therapy induces and/or maintains remission of active UC. Patients with chronic mild-to-severe relapsing UC were randomly assigned to oral amoxicillin 1500 mg/day, tetracycline 1500 mg/day, and metronidazole 750 mg/day, vs. placebo, for 2 weeks, and then followed up. The primary study end point was clinical response (Mayo score at 3 months after treatment completion) and secondary end points were clinical and endoscopic score improvements at 12 months. Anti-F. varium antibodies were measured by enzyme-linked immunosorbent assay. Treatment and placebo groups each had 105 subjects. At the primary end point, response rates were significantly greater with antibiotics than with placebo (44.8 vs. 22.8%, P=0.0011). Endoscopic scores significantly improved at 3 months (P=0.002 vs. placebo). Remission rates were 19.0% (antibiotics) vs. 15.8% (placebo) at 3 months (P=0.59). At the secondary end point, response rates were significantly greater with antibiotics than with placebo (49.5 vs. 21.8%, respectively, P<0.0001). Endoscopic scores were significantly improved at 12 months after antibiotic treatment (P=0.002 vs. placebo). Remission rates had improved to 26.7% with antibiotics vs. 14.9% for placebo, at 12 months (P=0.041). F. varium antibody titers decreased in responders but not in nonresponders, and more in the antibiotic than in the placebo group. More pretreatment steroid-dependent UC patients discontinued corticosteroids after treatment completion (6 months: 28.6 vs. 11.8%, respectively, P=0.046; 9 months: 34.7 vs. 13.7%, respectively, P=0.019; and 12 months: 34.7 vs. 13.7%, respectively, P=0.019). These effects were greater in the subanalysis of the active group (Mayo scores of 6-12) than in that of total cases (0-12). No serious drug-related toxicities occurred. The 2-week triple antibiotic therapy produced improvement, remission, and steroid withdrawal in active UC more effectively than a placebo.

Luminol-dependent chemiluminescence of peripheral neutrophils in asthmatic patients

To evaluate the role of the neutrophil in the pathophysiology of bronchial asthma, we examined the luminol-dependent chemiluminescence of peripheral neutrophils (CL) in steroid-dependent asthmatic patients during an acute attack (n = 18), during stable period (n = 10), and in normal controls (n = 21). When stimulated with formyl-methionyl-leucyl-phenylalanine (fMLP 0.1 microM), the CL of patients in acute attack was lower than that of normal controls. (5.29 +/- 1.25 vs. 9.29 +/- 0.84 relative light units p less than 0.01) Among 18 patients, we could examine 10 patients during the stable period after receiving steroids. The CL of those 10 patients during the stable period was significantly higher than their CL during acute attacks, when evaluated by the paired t-test (p less than 0.05), and was not very different than in normal controls. When stimulated with opsonized zymosan (5 mg/ml), CL was similar in the three groups. We also examined the CL of peripheral neutrophils of normal persons after the preincubation with serum of patients during acute attack and stable period. When stimulated with fMLP, CL after preincubation with serum of patients during acute attack was lower than CL after preincubation with the serum of patients during the stable period. It was suggested that the respiratory-burst activity of peripheral neutrophils in acute asthmatic attack was reduced to the chemotactic stimulus of fMLP.

Rituximab for Multirelapsing, Steroid-Dependent or Steroid-Resistant, Minimal-Change Nephropathy – a Report of Nine Adult Cases

Minimal-change nephropathy (MCN) accounts for 10–15% of cases of the nephrotic syndrome in adults. Frequent relapses occur in 10–25% of cases and steroid dependence is seen in 25–30%. Rituximab is a chimeric mono-clonal antibody directed against CD20-positive B-cells with reported benefit in immune-mediated renal disease. We present six female and three male patients, ranging from 27–70 years of age, with multirelapsing, steroid-dependant or steroid-resistant MCN, with previous multiple courses of corticosteroids (CS) and second-line therapies. Five patients were steroid dependant with the lowest daily CS dose between 5–20 mg/day. All patients were given two doses of rituximab, each a flat dose of 500 mg except for one case who received a dose of 1000 mg. All patients were B-cell depleted before the second dose. No major side effects occurred. Seven of the patients responded to therapy with normalized plasma albumin and no albuminuria enabling discontinuation or tapering of CS significantly below levels where relapses had occurred in the past (P=0.03). One patient reached partial remission and another had no response to therapy. Follow-up was 6–33 months. There were three relapses after 9, 12, and 20 months, respectively. In conclusion, frequency of relapses and steroid dependency or resistance in MCN remain ther-apeutic challenges to physicians. The current case series suggests that B-cell depletion may induce long-lasting remissions and, as a result, has a potential as a steroid-sparing agent in the treatment of steroid-dependent and multire-lapsing MCN patients.

Anatomy, pathology, and physiology of the tracheobronchial tree: Emphasis on the distal airways

This article covers the airway tree with respect to anatomy, pathology, and physiology. The anatomic portion discusses various primate groups so as to help investigators understand similarities and differences between animal models. An emphasis is on distal airway findings. The pathology section focuses on the inflammatory responses that occur in proximal and distal airways. The physiologic review brings together the anatomic and pathologic components to the functional state and proposes ways to evaluate the small airways in patients with asthma.

Long-Term Outcome of Children with Steroid-Sensitive Idiopathic Nephrotic Syndrome

In children, the most frequent glomerular disease is idiopathic nephrotic syndrome, which is defined by the combination of a nephrotic syndrome and nonspecific histologic abnormalities of the kidney including minimal changes, FSGS, and diffuse mesangial proliferation. The term minimal-change disease has become synonymous with steroid-sensitive idiopathic nephrotic syndrome, although renal biopsy is usually not performed for patients who respond to steroid therapy. Indeed, the response to steroid therapy carries a greater prognostic weight than the histologic features seen on initial renal biopsy. Up to 80% of children with idiopathic nephrotic syndrome respond to corticosteroids, with a complete remission occurring within 30 d. Approximately one third of these patients have only one attack and are definitively cured after the course of corticosteroids. Ten to 20% of patients experience relapses several months after stopping the treatment and a cure takes place after three or four episodes, which respond to a standard course of corticosteroids. The remaining 40 to 50% of patients experience frequent relapses either as soon as steroid therapy is stopped (frequent relapsers) or when the dosage of steroids is decreased (steroid dependent). These steroid-dependent patients may have a prolonged course. In the long term, the risk for relapse and the …

Appropriateness of therapy for active Crohn's disease: Results of a multidisciplinary international expert panel—EPACT II

The increasing number of trials testing management strategies for luminal Crohn's disease (CD) has not filled all the gaps in our knowledge and thus, in clinical practice, many decisions for CD patients have to be taken without the benefit of high-quality evidence. Methods A multidisciplinary European expert panel used the RAND Appropriateness Method to develop and rate explicit criteria for the management of individual patients with active, steroid-dependent (ST-D) and steroid-refractory (ST-R) CD. Results Overall, 296 indications pertaining to mild-to-moderate, severe, ST-D, and ST-R CD were rated. In anti-TNF naïve patients, budesonide and prednisone were found to be appropriate for mild-moderate CD, and infliximab (IFX) was appropriate when these had previously failed or had not been tolerated. In patients with a prior successful treatment by IFX, this drug, with or without co-administration of a thiopurine analog, was favoured. Other anti-TNFs were appropriate in the presence of intolerance or resistance to IFX. High-dose steroids, IFX or adalimumab were appropriate in severe active CD. For the 105 indications for ST-D or ST-R disease, the panel considered the thiopurine analogs, methotrexate, IFX, adalimumab, and surgery for limited resection, to be appropriate, depending on the outcome of prior therapies. Anti-TNFs were generally considered appropriate in ST-R. Conclusion Steroids, including budesonide for mild-to-moderate CD, remain the first-line therapy for active luminal CD. Anti-TNFs, in particular IFX as shown by the amount of available evidence, remain the second-line therapy for most indications. Thiopurine analogs, methotrexate and anti-TNFs are favoured in ST-D patients and ST-R patients.

Treatment of steroid-naive ulcerative colitis

The introduction of steroid therapy by Truelove and Witts in the 1950s revolutionized the treatment of ulcerative colitis. Corticosteroids are potent inhibitors of T-cell activation and proinflammatory cytokines and still represent the mainstay of therapy of patients with ulcerative colitis. About 15% of patients are resistant to steroids, and about a quarter of patients become dependent within 1 year of therapy. Steroid-related adverse events are numerous and occur frequently. So, new steroids with low systemic absorption and better safety profile have been studied, but they show an overall lower efficacy compared with traditional steroids. A new drug-delivery system based on the use of autologous erythrocytes loaded with dexamethasone 21-phosphate has been recently developed. Several studies have demonstrated its efficacy in steroid-dependent patients leading to complete withdrawal of oral steroids and disappearance of the most steroid-related adverse events. In this review we elaborate on the role of steroids in the treatment of ulcerative colitis, focusing on the aspects related to the mechanisms of action and resistance to the steroids, and their secondary effects. Moreover, we analyse the alternatives to traditional systemic steroids such as the new steroids with low bioavailability and the steroids encapsulated into erythrocytes.

Long Term Use of Glucocorticoid Induced Osteoporotic Multiple Compression Fractures in Autoimmune Diseases

Reduced bone mineral density precedes the development of vertebral fractures in patients under long term glucocorticoid therapy. Osteoporosis is a frequent complication in steroiddependent patients, and the risk of developing vertebral fractures in these patients is much higher than involutional osteoporosis. We described a 54-year-old patient who presented with autoimmune hepatitis and had a 6-year history of steroid medication. The patient had multiple compression fractures (T10∼L5) without trauma, and was treated successfully with multi-level vertebroplasty and an intravenous injection of bisphosphonate without complications.

Emergent and Elective Surgery for Diverticulitis

The scientific evidence and basis for standardized treatment for diverticulitis has been questioned. For years, medical and surgical management of acute diverticulitis included the theory that more than 2 significant attacks of diverticulitis would lead to the recommendations of surgical resection. This should be questioned and further investigated with prospective randomized trials. Only a small number of well-published articles support the surgical management with good scientific data. Although our ability to take a history and skill of physical examination has not changed, the use of improved technology such as high-speed computerized axial tomography has afforded us the ability to make earlier and more accurate diagnoses. This may further allow us to standardize treatment and study outcomes. It is possible that only the most critical situations may necessitate an operation. The age group less than 40 years, the immunocompromised, steroid-dependent, diabetic, and transplant patients, seem to be at greater risk with increased morbidity if not treated early and aggressively. Those individuals who present with perforation or compromised obstruction most likely will continue to need emergent intervention. But we need to reevaluate who needs surgical intervention while remaining within the confines of excellent and cost-effective care.

Clinical Outcomes of Children with IBD with Unfavorable Thiopurine Metabolism

Purpose: There are well-established data supporting the use of immunomodulatory therapy with the thiopurine agents azathioprine (AZA) or 6-mercaptopurine (6-MP) for the treatment of patients with inflammatory bowel disease (IBD). A poor clinical response to thiopurine can be seen in patients with preferential production of 6-MMP, an inactive thiopurine metabolite, and associated low 6-TGN levels. The use of allopurinol in 6-MP or AZA-treated adults with preferential 6-MMP production increases levels of 6-TGN while reducing 6-MMP. We describe the clinical outcomes of children with IBD who were treated with allopurinol because of preferential metabolism of AZA/6-MP to 6-MMP rather than 6-TGN. Methods: A retrospective chart review was performed. Subjects were poor responders to AZA/6-MP and had elevated 6-MMP metabolite levels. All were started by their clinicians on allopurinol and had their dose of AZA/6-MP reduced to 25–50% of their initial dose. A clinical goal was defined at the beginning of therapy for each subject, and response was defined as successfully achieving the goal by 3 months after initiating allopurinol. In addition, lab values (6-TGN, 6-MMP, WBC and ALT) were compared before and 3 months after starting allopurinol. Disease activity was categorized by physician's global assessment (PGA). Results: 12 children (ages 7–18 yrs, 8 males, 11 Crohns, 1 UC) met inclusion criteria. Indications for treatment included active disease (5) and increased transaminases (7). Lab values pre- and post-allopurinol are summarized in the Table. The addition of allopurinol led to achieving clinical goals in 10/12 subjects. One subject with high ALT did not respond, and 1 discontinued treatment due to leukopenia. A third initially had an improved PGA, but required resection 4 months after starting allopurinol. 4/4 steroid dependent patients discontinued steroids after starting allopurinol.Table: Laboratory Values Pre- and Post-Allopurinol.Conclusion: Allopurinol + reduced dose AZA/6-MP effectively optimizes 6-TGN levels and improves clinical outcomes in children whose metabolism promotes the production of 6-MMP. Hepatotoxicity can be reversed, and steroid dependence eliminated.

Steroid‐sparing effect of omalizumab for allergic bronchopulmonary aspergillosis and cystic fibrosis

Allergic bronchopulmonary aspergillosis (ABPA) is a complication commonly encountered in patients with CF that produces significant respiratory morbidity. Chronic airway colonization with Aspergillus induces strong inflammatory responses with high IgE levels. Current guidelines for therapy include prolonged courses of systemic corticosteroids as the main therapeutic strategy. However this has the potential to induce significant detrimental side effects in children. Omalizumab is a humanized monoclonal antibody directed against IgE that prevents its binding to high- and low-affinity receptors on effector cells. It has been shown to be effective in improving asthma control in patients with a strong allergic component. We present our long term experience with the use of Anti-IgE therapy in three children with CF and ABPA (mean age at start of therapy 14.2 years) who were steroid dependent. All three were already experiencing significant side effects from chronic steroid therapy. After the start of Omalizumab these children have experienced significant and sustained clinical improvements at the same time that they were discontinued from chronic systemic steroids. Our experience suggests that IgE blockade has tremendous potential as a strategy to control this disease in steroid dependent patients.

In patients with ulcerative colitis, adsorptive depletion of granulocytes and monocytes impacts mucosal level of neutrophils and clinically is most effective in steroid naïve patients

Background. The aetiology of ulcerative colitis is inadequately understood, and drug therapy has been empirical rather than based on sound understanding of disease aetiology. This has been a major factor for refractoriness and adverse drug effects as additional complications. However, ulcerative colitis by its very nature is exacerbated and perpetuated by inflammatory cytokines, which are released by peripheral granulocytes and monocytes as well. Additionally, active ulcerative colitis is often associated with elevated peripheral granulocytes and monocytes with activation behaviour and are found in vast numbers within the colonic mucosa. Hence, from the clinicopathologic viewpoint, granulocytes and monocytes are appropriate targets for therapy in ulcerative colitis. Based on this thinking, an Adacolumn has been developed for depleting excess granulocytes and monocytes by adsorption. Methods. By colonoscopy, biopsy and histology, we investigated the impact of granulocyte and monocyte adsorption (GMA) on the mucosal level of granulocytes and monocytes in patients with active ulcerative colitis. Forty-five patients (26 steroid naïve and 19 steroid-dependent), mean age 44.7 yr, were included. Twenty patients had total colitis and 25 had left-sided colitis. Each patient was given up to 11 GMA sessions over 12 weeks. No patient received additional medications within 4 weeks (steroid) to 8 weeks (other immunosuppressants) prior to entry or during the GMA course. Colonoscopy together with biopsy was done at entry and within 2 weeks after the last GMA session. Results. At entry, the mean clinical activity index was 12.6; range 10–16. A total of 400 colonic biopsies were examined, which revealed massive infiltration of the colonic mucosa by granulocytes, and GMA was associated with striking reduction of granulocytes in the mucosa. At week 12, 33 of 45 patients (73.3%, P < 0.01) had achieved clinical remission (the mean clinical activity index ≤ 4). Colonoscopy revealed that most non-responders had deep colonic ulcers and extensive loss of the mucosal tissue. The response rate in steroid naïve subgroup was 22 of 26 patients (84.6%, P < 0.005) and in steroid-dependent was 11 of 19 (57.9%, P < 0.05 and P = 0.02154 for steroid naïve vs. steroid-dependent). Patients who achieved remission could continue with their salicylates. On average, remission was sustained for 7.8 months in all 33 responders. Conclusions. This is the first report showing a striking difference in clinical response to GMA between steroid naïve and steroid-dependent patients. Further, patients with deep colonic ulcers together with extensive loss of the mucosal tissue are not like to respond to GMA.

Long-term Methotrexate for Crohn's Disease

To assess the efficacy and safety profile of methotrexate (MTX) for the treatment of Crohn's disease (CD) in clinical practice. MTX is widely used for some chronic immunologic diseases. Although some randomized controlled trials suggest its efficacy in CD, this drug remains a second-line, underused, immunomodulator. Medical records of all patients treated with MTX for CD in our center (n=44) were reviewed. Clinical and epidemiologic parameters, including risk factors for hepatotoxicity, were registered. MTX was prescribed mainly for steroid-dependency (n=22) and as concomitant treatment to infliximab (n=18). Mean duration of treatment was 22.9+/-19 months, with a mean cumulative dose of MTX of 1169+/-784 mg. Thirty-nine percent of patients developed drug-related side effects, hepatotoxicity being the most frequent [13 patients (30%)]. However, only 5 patients (11%) had to discontinue MTX. In steroid-dependent CD patients, disease remission and complete steroid withdrawal was achieved in 77% of cases. Seven patients lost their initial response to MTX during follow-up, leading to a cumulative probability of remission of 39% after 3 years of treatment. MTX is well tolerated in most CD patients. Although a great proportion of steroid-dependent CD patients achieve disease remission and steroid withdrawal, there is a trend to a loss of efficacy with time. Larger, long-term studies are necessary to establish the role of MTX in the management of CD.

921 Long Term Follow-Up of a Cohort of Steroid-Dependent Crohn's Disease Patients Included in a Randomized Trial Evaluating Short Term Infliximab Combined with Azathioprine

921 Long Term Follow-Up of a Cohort of Steroid-Dependent Crohn's Disease Patients Included in a Randomized Trial Evaluating Short Term Infliximab Combined with Azathioprine

The treatment with levamisole of frequently recurring steroid-sensitive idiopathic nephrotic syndrome in children

The treatment of frequently relapsing steroid-sensitive nephrotic syndrome in children with established immunosuppressive drugs (steroids, cyclophosphamide, cyclosporin A) sometimes presents problems because of the expected incidence of side effects. Stimulation of the immune system with the anthelminthic drug levamisole in this disease has been documented. Aim of this study was to assess in a prospective but uncontrolled series of observations its value and side effects. 25 patients (15 boys, ten girls; median age 10 [3.5-22] years) were given levamisole, 2 mg/kg/48 h. Before this treatment was started eight of the children/adolescents (32%) had frequent relapses and 17 (68%) had become steroid-dependent. Treatment was started during steroid-induced remission and continued for 3-24 (median 6) month, while steroids were discontinued after four weeks. Relapse frequency per patient month was reduced from a mean of 0.5 (0.33-0.83) before to 0.31 (0-0.67) during levamisole administration (P < 0.001). In 12 patients (48%) no or considerably fewer relapses were observed. Patients with exclusively frequent relapses responded to levamisole better than those with steroid dependence (7/8 [87.5%] vs. 5/18 [27.7%], P = 0.01). Side effects were reversible leukopenia in two patients and nonspecific skin rash as well as epigastric pain in one patient. Levamisole is an efficacious addition or alternative, with a low incidence of side effects, in the treatment of frequently relapsing nephrotic syndrome, particularly so in yet steroid-dependent patients.

Use of methotrexate in refractory Crohnʼs disease: The Edinburgh experience

In the two benchmark controlled trials in Crohn's disease (CD) supporting its use, methotrexate (MTX) was used as the immunosuppressant of choice in immunomodulatory-naive patients. However, in daily clinical practice MTX is used generally after thiopurine analogs have failed. The data are reported using intramuscular (IM) MTX (25 mg/week) in the induction of remission and oral MTX (15 mg/week) in 39 CD patients with a median age of 32 years, assessed retrospectively. In all, 97% patients had failed azathioprine and/or mercaptopurine therapy due to lack of efficacy in 14 (36%) and side effects in 24 (61%) patients; 21 patients (53%) were steroid-dependent with a median dose of 27.5 mg prednisolone/day for over a year. In all, 72% of patients tolerated an induction regimen of 25 mg/week of IM MTX; 10% managed a reduced dose and 18% were intolerant. Remission was achieved in 71% of patients at 16 weeks. In the patients taking corticosteroids, withdrawal was achieved in 26% of patients and reduction in 47% at 16 weeks. Oral MTX therapy was continued in 22 patients after induction. In this group the probability of relapse was 78% at 50 weeks of oral therapy. Parenteral MTX therapy is efficacious in inducing remission in steroid-dependent CD patients, although its use is limited by side effects in approximately 30% of patients. Low-dose oral therapy does not maintain long-term remission and is not a suitable alternative.

Clinical efficacy and cytokine network-modulating effects of tacrolimus in myasthenia gravis

To clarify the long-term efficacy, safety and the cytokine network-modulating effects of tacrolimus in myasthenia gravis, medical records of 86 newly diagnosed consecutive patients and nine steroid-dependent patients were retrospectively reviewed, and peripheral blood mononuclear cells (PBMC) were cultured for the cytokine profile. Steroid reduction effects were observed by using tacrolimus, and no serious adverse effects were observed. The culture study showed reduced IL-12, IL-17, IFN-γ, GM-CSF, TNF-α and MIP-1β, and elevated IL-10 in the PBMC from patients who received tacrolimus, which suggests inhibition of T cells and macrophages, and enhancement of type 1 regulatory T cells.

Efficacy and safety of dapsone as a second-line treatment in non-splenectomized adults with immune thrombocytopenic purpura

In adults with immune thrombocytopenic purpura (ITP), steroids are usually proposed as first-line therapy, but long-term complete responses are obtained in no more than 20% of patients. For the remaining patients, splenectomy is considered the treatment of choice, with reported “cure” rates from 60–70%. However, the inherent risks of surgery and sepsis after splenectomy without a guarantee of success justify the search for strategies aimed to avoid splenectomy. Here we retrospectively evaluated the results of dapsone treatment in ITP patients that failed first-line therapy with steroids. These patients received dapsone 100 mg/day for a minimum of 30 days before splenectomy was considered. Efficacy was defined as a sustained rise in platelet counts (>50 × 109/l) clearly attributed to dapsone treatment. Among 52 steroid-dependent or refractory patients, dapsone resulted in sustained increases in platelet counts in 44.2% of patients, after a median follow-up of 21.10 months after treatment initiation. The long-term efficacy of dapsone in this setting is further corroborated by the observation that none of the “responding” patients required splenectomy in the follow-up, compared to 69.0% of the “non-responding” patients. Dapsone-related adverse events were mild and promptly reversed by treatment withdrawal. The results of our retrospective analysis suggest that dapsone is a safe and effective second-line agent for steroid-dependent or refractory ITP patients. Because of its well-known safety profile and low cost compared to other potential second-line treatments for ITP, a trial course of dapsone should be viewed as an attractive option before splenectomy in steroid-dependent of refractory adult ITP patients.